Dwarfism, Madelung Deformity. NORD Database
Dyschondrosteosis is a very rare inherited disorder characterized by unusually shortened, bowed bones in the forearms (radius and ulna), abnormal deviation of the wrist toward the thumb side of the hand due to shortening of the radius and dislocation of the end portion of the ulna...
* chondrodysplasia of distal radial epiphysis
* (?) part of dyschondrosteosis (skeletal dysplasia)
* usually bilateral
* first apparent in early adolescence
* "bayonet"-shaped appearance of hand and wrist resembles anterior dislocation
Madelung deformity (MD) of the wrist is characterized by a growth disturbance in the volar-ulnar distal radial physis that results in a volar and ulnar tilted distal radial articular surface, volar translation of the hand and wrist, and a dorsally prominent distal ulna. It occurs predominantly in adolescent females who...
* Arrest of epiphyseal growth of the ulnar (medial) and volar (anterior) portions of the distal radius
* Leads to shortening of the radius and relative overgrowth of the ulna
* Inherited as an autosomal dominant due to mutation on X chromosome and has variable penetrance
- known as carpus curves, is result of epiphyseal arrest on ulnar & volar half of the distal radius, which causes articular surface to be directed ulnarward and volarward;
- genetics: it is inheritited as autosomal dominant trait w/ variable expressivity;
- females are affected four times as often as males
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